Ma C, Ow A, Shan O H, Wu Y, Zhang C, Sun J, Ji T, Pingarron Martin L, Wang L
Department of Oral and Maxillofacial Surgery - Head and Neck Oncology, 9th People's Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai Key Laboratory of Stomatology, Shanghai, China.
Department of Oral and Maxillofacial Surgery, Faculty of Dentistry, National University of Singapore, Singapore.
Int J Oral Maxillofac Surg. 2014 Aug;43(8):924-32. doi: 10.1016/j.ijom.2014.03.006. Epub 2014 Mar 28.
Malignant peripheral nerve sheath tumours (MPNST) are rare soft tissue sarcomas. The aim of this study was to assess clinicopathological characteristics and prognostic factors in order to improve the treatment of such tumours in the head and neck region. We performed a retrospective analysis of head and neck MPNST patients in our hospital between 1996 and 2012. Clinical features and pathological findings of these cases (n=43) were summarized. In addition, prognostic variables were evaluated by univariate and multivariate analyses. The median age of the patients at presentation was 41 years. Surgery was the main treatment approach. Pertinent information regarding the presence of neurofibromatosis type 1 was found in 13 patients (30.2%). Two-thirds of these patients were admitted for a primary tumour (n=27, 62.8%), while one-third (n=16, 37.2%) were treated for recurrent neoplasms. The overall survival rate was 46.5%. Multivariable analysis identified tumour size, surgical margins, and postoperative radiotherapy to be independent prognostic factors. MPNST of the head and neck is extremely difficult to manage. Surgery with postoperative radiation may be the optimum choice of treatment for primary head and neck MPNST.
恶性外周神经鞘瘤（MPNST）是一种罕见的软组织肉瘤。本研究旨在评估临床病理特征和预后因素，以改善对头颈部此类肿瘤的治疗。我们对1996年至2012年间我院收治的头颈部MPNST患者进行了回顾性分析。总结了这些病例（n = 43）的临床特征和病理结果。此外，通过单因素和多因素分析评估预后变量。患者就诊时的中位年龄为41岁。手术是主要的治疗方法。在13例患者（30.2%）中发现了与1型神经纤维瘤病相关的信息。这些患者中有三分之二因原发性肿瘤入院（n = 27，62.8%），而三分之一（n = 16，37.2%）接受复发性肿瘤治疗。总生存率为46.5%。多变量分析确定肿瘤大小、手术切缘和术后放疗是独立的预后因素。头颈部MPNST极难治疗。手术联合术后放疗可能是原发性头颈部MPNST的最佳治疗选择。
